Paeds · cardiology
Coarctation and interrupted aortic arch
Also known as Coarctation of the aorta · Interrupted aortic arch · IAA · Ductal-dependent arch obstruction · Aortic isthmus stenosis · Hypoplastic aortic arch
Fellowship guide to coarctation of the aorta and interrupted aortic arch: the two ductal-dependent left-heart-outflow obstructions that declare themselves as neonatal collapse when the duct closes, the four-limb blood-pressure gradient and weak femoral pulses at the bedside, prostaglandin E1 as the non-negotiable bridge to surgery, and the syndromic associations (Turner, 22q11.2, bicuspid aortic valve) that every candidate must name.
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Red flags
- A neonate who was well for the first days of life then presents in shock with metabolic acidosis and weak or absent femoral pulses has a ductal-dependent arch obstruction until proven otherwise — start prostaglandin E1 immediately, do not wait for the echocardiogram
- In any hypertensive child or adolescent, always measure four-limb blood pressures: an arm-to-leg systolic gradient above twenty millimetres of mercury with weak or delayed femoral pulses is coarctation until excluded
- A girl with coarctation of the aorta has Turner syndrome until a karyotype proves otherwise — request chromosomal analysis, because the cardiovascular and gonadal management both depend on it
- An interrupted aortic arch, especially type B distal to the left subclavian, is strongly associated with 22q11.2 deletion (DiGeorge syndrome) — test by fluorescence in-situ hybridisation or chromosomal microarray, as it changes the surgical airway, calcium and immunology plan
- Prostaglandin E1 apnoea is common and often requires intubation — an infant on a PGE1 infusion who is being transported must travel intubated or with an anaesthetist, because apnoea in transit is a preventable death
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- Coarctation of the aorta
- Interrupted aortic arch
- Ductal-dependent congenital heart disease
- Arch obstruction: coarctation and IAA
- Neonatal cyanosis and collapse
- Short case: coarctation of the aorta
- Long case: post-coarctation repair follow-up
- Coarctation and IAA
- Ductal-dependent circulation
- Cardiovascular examination: femoral pulses and four-limb BP
- Patient Care: congenital heart disease
- Medical Expert: coarctation of the aorta
The single idea that organises the whole topic is that coarctation and IAA are the same disease at different severities: both obstruct the aortic arch, both depend on the duct for descending-aortic flow, and both declare themselves at ductal closure. Coarctation narrows the isthmus; IAA interrupts it. The clinical picture, the resuscitation and the surgical logic flow from that one mechanism. [1] [6]
This page covers the recognition and neonatal management of both lesions, the syndromic associations that change the work-up (Turner syndrome, 22q11.2 deletion, bicuspid aortic valve), the surgical options and their complications, and the lifelong follow-up that every repaired patient needs. It links to the ductal-dependent congenital heart disease and neonatal cyanosis leaves for the broader differential. [2] [5]
Overview & Definition
Coarctation of the aorta is a discrete shelf-like infolding of the media at the aortic isthmus — the segment between the left subclavian artery and the ductus arteriosus — that narrows the lumen and obstructs forward flow to the descending aorta. Interrupted aortic arch is the extreme of the same spectrum: the arch is completely discontinuous, so the descending aorta receives blood only through the ductus arteriosus. [1] [2]
What sets these lesions apart from other left-heart obstructions is their ductal dependence. In fetal life the duct carries right ventricular output to the descending aorta, bypassing the narrowed or interrupted isthmus entirely, so the fetus grows normally and the lesion is invisible at birth. The disease declares itself only when the duct constricts over the first hours to days of life — suddenly cutting off the only route to the descending aorta and causing acute inferior-body hypoperfusion. This is why the classic neonatal coarctation or IAA presentation is a well baby who collapses on day three or four. [3] [4]
The two lesions sit within the broader family of ductal-dependent congenital heart disease, alongside hypoplastic left heart syndrome, critical aortic stenosis and transposition. The unifying principle is that the neonate is stable only while the duct is open, so prostaglandin E1 is the first drug in any neonate who collapses after a period of wellbeing and shows shock, differential cyanosis or weak pulses. [4] [7]
References12ShowHide
- [1]Salciccioli KB; Zachariah JP Coarctation of the Aorta: Modern Paradigms Across the Lifespan. Hypertension, 2023.PMID 37476999
- [2]Kim YY; Andrade L; Cook SC Aortic Coarctation. Cardiol Clin, 2020.PMID 32622489
- [3]Hede SV; DeVore G; Satou G; et al Neonatal management of prenatally suspected coarctation of the aorta. Prenat Diagn, 2020.PMID 32277716
- [4]Bansal N; Balakrishnan PL; Aggarwal S Prostaglandin Infusion in Neonate With Severe Coarctation of the Aorta With Closed Ductus Arteriosus — A Case Report and Review of the Literature. World J Pediatr Congenit Heart Surg, 2020.PMID 31010402
- [5]LaPar DJ; Baird CW Surgical Considerations in Interrupted Aortic Arch. Semin Cardiothorac Vasc Anesth, 2018.PMID 29774793
- [6]Schreiber C; Mazzitelli D; Haehnel JC; et al The interrupted aortic arch: an overview after 20 years of surgical treatment. Eur J Cardiothorac Surg, 1997.PMID 9332928
- [7]Burbano-Vera N; Zaleski KL; Latham GJ; et al Perioperative and Anesthetic Considerations in Interrupted Aortic Arch. Semin Cardiothorac Vasc Anesth, 2018.PMID 29742969
- [8]Ron HA; Crowley TB; Liu Y; et al Improved Outcomes in Patients with 22q11.2 Deletion Syndrome and Diagnosis of Interrupted Aortic Arch Prior to Birth Hospital Discharge, a Retrospective Study. Genes (Basel), 2022.PMID 36672801
- [9]Eckhauser A; South ST; Meyers L; et al Turner Syndrome in Girls Presenting with Coarctation of the Aorta. J Pediatr, 2015.PMID 26323199
- [10]Wu Y; Jin X; Kuang H; et al Is balloon angioplasty superior to surgery in the treatment of paediatric native coarctation of the aorta: a systematic review and meta-analysis. Interact Cardiovasc Thorac Surg, 2019.PMID 30060099
- [11]Toro-Salazar OH; Steinberger J; Thomas W; et al Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol, 2002.PMID 11867038
- [12]Sinning C; Zengin E; Kozlik-Feldmann R; et al Bicuspid aortic valve and aortic coarctation in congenital heart disease — important aspects for treatment with focus on aortic vasculopathy. Cardiovasc Diagn Ther, 2018.PMID 30740325